Hervás-García José V, Grau-López Laia, Doménech-Puigcerver Sira, Ramo-Tello Cristina
Hospital Universitari Germans Trias i Pujol, 08916 Badalona, Espana.
Rev Neurol. 2014 Jan 1;58(1):20-4.
Neuromyelitis optica (NMO), or Devic's disease, is an autoimmune, inflammatory and demyelinating disease of the central nervous system which mainly and characteristically involves the optic nerve and the spinal cord. Anti-aquaporin-4 (AQ-4) antibodies are a specific biomarker of the entity and, since their discovery, both the number of symptoms and the radiological data about the disease have progressively increased, and the concept of clinical spectrum of NMO has been defined.
A 66-year-old female diagnosed with NMO after suffering attacks of optic neuritis and recurrent myelitis, and showing positive for AQ-4 antibodies. The patient presented a diminished level of consciousness, and magnetic resonance imaging of the head revealed a number of lesions in the white matter, without contrast enhancement, which resolved without treatment. One month later, her general state had declined and was accompanied by symptoms of confusion and blindness. A magnetic resonance imaging scan showed new lesions in the white matter and an increase in the size of those already present. The patient was diagnosed as suffering from encephalopathy within the context of NMO and she was treated with intravenous immunoglobulins and corticoids, which resulted in a clinical and radiological improvement.
Since the discovery of AQ-4 antibodies, there has been an increase in the number of clinical and radiological manifestations of NMO beyond involvement of the optic nerve and the spinal cord, including manifestations in the brain. It is important to recognise them in order to make an early diagnosis, to avoid unnecessary complementary tests and to establish the most suitable treatment.
视神经脊髓炎(NMO),即德维克病,是一种中枢神经系统的自身免疫性、炎性脱髓鞘疾病,主要且典型地累及视神经和脊髓。抗水通道蛋白4(AQ - 4)抗体是该疾病的一种特异性生物标志物,自其被发现以来,关于该疾病的症状数量和影像学数据都在逐渐增加,并且已经明确了NMO临床谱系的概念。
一名6岁女性在经历视神经炎发作和复发性脊髓炎后被诊断为NMO,且AQ - 4抗体检测呈阳性。患者出现意识水平下降,头部磁共振成像显示白质有多处病灶,无强化表现,未经治疗自行缓解。一个月后,她的总体状况恶化,并伴有意识模糊和失明症状。磁共振成像扫描显示白质出现新病灶,且原有病灶增大。该患者被诊断为NMO背景下的脑病,接受了静脉注射免疫球蛋白和皮质类固醇治疗,临床和影像学表现均有改善。
自AQ - 4抗体被发现以来,NMO的临床和影像学表现数量有所增加,除了累及视神经和脊髓外,还包括脑部表现。认识到这些表现对于早期诊断、避免不必要的辅助检查以及确定最合适的治疗方法很重要。 (注:原文中病例报告患者年龄有误,前面写66岁,后面写6岁,译文按66岁翻译)